Hawrylkiewicz I, Izdebska-Makosa Z, Grebska E, Zielinski J
Bull Eur Physiopathol Respir. 1982 May-Jun;18(3):403-10.
We studied pulmonary haemodynamics in 34 patients with idiopathic pulmonary fibrosis (IPF). The diagnosis was established clinically and confirmed by a lung biopsy in 13 subjects. At rest pulmonary hypertension was found in 21 patients, the mean pulmonary artery pressure (PPA) for the group being 23.8 +/- 7.7 mmHg (means +/- SD). In 19 patients subjected to steady-state physical exercise, PPA rose from 23.1 +/- 7.7 mmHg to 40.3 +/- 16.6 mmHg and arterial oxygen tension (PaO2) fell from 77.7 +/- 10.1 mmHg to 62.5 +/- 14.3 mmHg. There was a significant correlation between the rise in PPA and fall in PaO2 on exercise. We found no correlation between pulmonary artery pressure referred to barometric pressure at rest and lung function data, except for a weak correlation between PPA and PaO2. However, pulmonary artery transmural pressure correlated more strongly with PaO2, and additional correlation with VC, FEV1, and lung compliance were found. Our results suggest that pulmonary intravascular pressure and pulmonary hypertension in patients with IPF are affected not only by anatomical restriction of the pulmonary vasculature and alveolar hypoxia but also by the negative intrathoracic pressure.
我们对34例特发性肺纤维化（IPF）患者的肺血流动力学进行了研究。诊断通过临床确立，并在13例患者中经肺活检证实。静息状态下，21例患者存在肺动脉高压，该组患者的平均肺动脉压（PPA）为23.8±7.7 mmHg（均值±标准差）。19例进行稳态体育锻炼的患者，PPA从23.1±7.7 mmHg升至40.3±16.6 mmHg，动脉血氧分压（PaO2）从77.7±10.1 mmHg降至62.5±14.3 mmHg。运动时PPA的升高与PaO2的降低之间存在显著相关性。我们发现静息状态下相对于大气压的肺动脉压与肺功能数据之间无相关性，除了PPA与PaO2之间存在微弱相关性。然而，肺动脉跨壁压与PaO2的相关性更强，并且还发现与肺活量（VC）、第一秒用力呼气容积（FEV1）和肺顺应性存在额外的相关性。我们的结果表明，IPF患者的肺血管内压和肺动脉高压不仅受肺血管解剖学限制和肺泡缺氧影响，还受胸内负压影响。
